Iris cyst and atypical complications / Cisto de íris e complicações atípicas

ABSTRACT The authors report the case of a male adult presenting significant ocular complications and irreversible visual impairment, resulting from the long-term progression and late diagnosis of an iris cyst in the right eye, probably secondary to trauma. The patient was admitted to Hospital Universitário Antonio Pedro with a total corneal opacity that blocked direct visualization of the anterior chamber. Ultrasound biomicroscopy was crucial for the anatomic study, and the patient was submitted to enucleation for aesthetic improvement and clarifying diagnosis. We concluded athalamia and deformation of the anterior segment, due to expansion of the cyst, led to gradual elevation of the intraocular pressure and damage of the optic nerve, resulting in visual loss.

RESUMO Relatamos o caso de um paciente com evolução e diagnóstico tardios de cisto de íris no olho direito, provavelmente secundário a trauma, com complicações importantes e baixa irreversível da visão, tendo sido admitido no Hospital Universitário Antônio Pedro já com leucoma total da córnea e câmara anterior indevassável. A biomicroscopia ultrassônica se mostrou imprescindível para o estudo anatômico, sendo o paciente finalmente submetido à enucleação, para melhora estética e elucidação diagnóstica. Concluímos que a atalamia e a desestruturação do segmento anterior, consequentes ao crescimento cístico, levaram a um gradativo aumento da pressão intraocular e lesão do nervo óptico, com consequente perda da visão.

Manejo anestésico de resección de quiste de vallécula en neonato. A propósito de un caso / Anesthetic management of vallecular cyst resection in neonate. A case report

Difficult airway management is one of the most important challenges an anesthesiologist faces. It is due to the high morbidity and mortality that it entails. The challenge is even greater if the patient is a newborn. For this reason, we should have different strategies that allow us to anticipate and treat possible complications derived from the procedure. In this case, we present a newborn with vallecular cyst and respiratory distress who is admitted for cyst resection. The gold-standard in anticipated difficult airway management is the fibrobronchoscope. We decided to perform an alternative management by means of orotracheal intubation with videolaryngoscope (Glydescope®) in spontaneous ventilation

El manejo de una vía aérea difícil es uno de los retos más importantes a los que puede enfrentarse un anestesiólogo debido a la elevada morbimortalidad que conlleva. El reto aún es mayor si el paciente es un neonato. Por este motivo, debemos contar con diferentes estrategias que permitan anticipar y poder tratar las posibles complicaciones derivadas del procedimiento. En este caso, presentamos un neonato con quiste de vallécula con clínica de trabajo respiratorio que es admitido para cirugía de exéresis del quiste. El gold standard en el manejo de una vía aérea difícil conocida es el fibrobroncoscopio. Nosotros decidimos realizar un manejo anestésico alternativo mediante intubación orotraqueal con videolaringoscopio (Glydescope®) en ventilación espontánea.

Enfermedad quística adventicial de la arteria poplítea: presentación imagenológica de una causa rara de claudicación intermitente / Adventitial cystic disease of the popliteal artery as a cause of intermittent claudication: not your usual suspect: report of one case

Adventitial cystic disease of the popliteal artery is a rare condition of uncertain etiology, which presents as intermittent claudication of the lower extremity in middle-age patients. We report a 44-year-old man presenting with intermittent claudication of his left leg. MR angiography showed cystic parietal lesions that caused compression with partial occlusion of the left popliteal artery. Surgical resection of the affected segment was performed, with venous graft interposition. The histopathological analysis of the surgical specimen was consistent with cystic adventitial disease.

Squamous cell carcinoma arising in a sacrococcygeal tailgut cyst

Abstract: We report a case of squamous cell carcinoma originated from a sacrococcygeal tailgut cyst in a 73-year-old female patient. Tailgut cysts are generally multilocal and have a layer of either columnar, squamous or transitional epithelium, or a combination of these. This case was treated with surgical excision and radiotherapy. Cancer presentation of a congenital abnormality in old age is a rare entity. This report is the first case of squamous cell carcinoma developing in a tailgut cyst without any synchronization, as an isolated (pure) pathology.

Glaucoma due to ciliary body cysts and pseudoplateau iris: a systematic review of the literature / Glaucoma associado a cistos de corpo ciliar e íris pseudoplateau: revisão sistemática da literatura

ABSTRACT The diagnosis of angle-closure glaucoma secondary to iridociliary cysts is challenging and lacks compiled literature support. We present a rare case of bilateral angle-closure glaucoma associated with pseudoplateau iris due to multiple ciliary cysts and conducted a systematic review of the literature to find similar case reports published between November 2006 and November 2016. Only 19 case reports present treatment modalities, and most cases required more than one therapeutic approach for controlling the intraocular pressure. Pseudoplateau iris attributed to iridociliary cysts should be considered in the differential diagnosis of patients with narrow angles, particularly those with ocular hypertension and glaucoma, in which management is complex. In addition to gonioscopy, ultrasound biomicroscopy is considered the conclusive method for accurate diagnosis.

RESUMO O diagnóstico de glaucoma de ângulo fechado secundário a cistos iridociliares é desafiador e não possui suporte da literatura compilada. Apresentamos um caso bilateral raro de glaucoma de ângulo fechado associado à íris pseudoplateau devido a cistos ciliares múltiplos e realizamos uma revisão sistemática da literatura de relatos de casos similares publicados entre novembro de 2006 e novembro de 2016. Apenas 19 relatos de casos apresentaram as modalidades de tratamento e na maioria deles foi necessário mais de uma abordagem terapêutica para controlar a pressão intra-ocular. Íris pseudoplateau atribuída a cistos iridociliários deve ser considerada no diagnóstico diferencial de pacientes com ângulos estreitos, particularmente aqueles com hipertensão ocular e glaucoma, em que o manejo é complexo. Além da go nioscopia, a biomicroscopia ultra-sônica é considerada o método conclusivo para o diagnóstico correto.

Quiste de primer arco branquial, reporte de caso y revisión de la literatura / First branchial arch cyst, case report and review of the literature

Los quistes branquiales de primer arco son una malformación congénita de baja frecuencia. Se desarrollan por la fusión incompleta de la hendidura faríngea entre el primer y el segundo arco branquial. Pueden permanecer silentes por mucho tiempo y manifestarse cuando se sobreinfectan, causando significativa morbilidad. Por lo general, se presentan en la infancia, pero muchas veces su diagnóstico es tardío ya que son difíciles de diferenciar de adenopatías u otras masas, debiendo ser considerados dentro del diagnóstico diferencial de masa cervical y parotídea, especialmente en pacientes jóvenes. La cirugía es el tratamiento definitivo, requiriendo una escisión completa del quiste y su trayecto fistuloso para evitar las recurrencias. Debido a su localización intra-parotídea y complejidad morfológica, es necesario conocer la anatomía en detalle para evitar lesionar el nervio facial. En esta revisión, se pretende presentar las características clínicas y resultados quirúrgicos en una paciente lactante con diagnóstico de quiste de primer arco, asícomo efectuar una revisión de la literatura.

First branchial arch cysts are very infrequent congenital malformations. Their development is due to an incomplete fusion of the pharyngeal cleft between the first and second branchial arch. They can remain silent for a very long time and manifest when they become infected, causing significant morbidity. In general, they present in child-hood, but many times its diagnosis occurs on a late onset since they are difficult to differentiate from lymphadenopathies or other masses. So, they should be considered on the differential diagnosis of cervical and parotid masses, especially in young patients. Surgery is its definitive treatment. Complete excision of the cyst and its fistula must be performed to avoid recurrences. Due to its intraparotid localization and morphological complexity it is necessary to understand the anatomy in detail to avoid facial nerve le-sions. In this revision, we aim to present clinical characteristics and surgical outcomes in an infant with the diagnosis of first branchial arch cyst and to review the literature.

Quiste de vallécula como causa de estridor congénito: reporte de un caso y revisión de la literatura / Vallecula cyst as a cause of congenital stridor: case report and literature review

El quiste de vallécula congénito es una malformación de vía aérea poco frecuente que se manifiesta principalmente con estridor laríngeo y que puede plantear problemas diagnósticos y terapéuticos complejos, a menudo en situaciones de riesgo vital. Se presenta el caso de un recién nacido de pretérmino (RNPT) de 36 semanas pequeño edad gesta-cional (PEG), portador de un estridor laríngeo congénito y mal incremento pondoestatural que en relación a una infección respiratoria baja, presentó empeoramiento del estridor laríngeo a los 48 días de edad cronológica. Por insuficiencia respiratoria aguda requirió de intubación orotraqueal que no resultó dificultosa. La extubación fue fallida por presentar estridor inspiratorio franco. La nasofibroscopía demostró una lesión de aspecto quístico en base de lengua que desplazaba la epiglotis hacia posterior obstruyendo parcialmente el lumen de la vía aérea. Por laringoscopía directa se realizó marsupialización. Se realiza la revisión bibliográfica y se analiza el caso y su tratamiento.

Congenital vallecular cyst is a rare airway malformation mainly manifested by laryngeal stridor and could generate complex diagnostic and therapeutic problems, often in life-threatening situations. We present the case of a pre-term newborn of 36 weeks small for gestational age, who at 48 days of chronological age showed worsening of a congenital laryngeal stridor in the context of a lower respiratory tract infection associated to low weight gain from birth. For reasons of acute respiratory failure, orotracheal intubation was executed which was not difficult. Extubation was failed because the child presents significant inspiratory stridor. Nasofibroscopy showed a cystic lesion of the tongue base that pushed backward the epiglottis obstructing partially the airway lumen. Marsupialization was performed by direct laryngoscopy. A Bibliographic review was done and the case and its treatment are discuss.

Influence of vascular endothelial growth factor inhibition on simple renal cysts in patients receiving bevacizumab-based chemotherapy

PURPOSE: Although angiogenesis has been implicated in the promotion of renal cyst growth in autosomal dominant polycystic kidney disease, no studies have investigated the role of angiogenesis in the growth of simple renal cysts. The aim of current study was to investigate the effect of chemotherapy with the antivascular endothelial growth factor antibody bevacizumab on renal cyst development and growth in cancer patients. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 136 patients with a variety of cancers that were treated with bevacizumab-based chemotherapy for metastatic disease. The presence of and changes in renal cysts were evaluated by retrospective analysis of computed tomography scans performed for assessment of tumor response to bevacizumab-based therapy. RESULTS: The median age of the patients was 64 years. Renal cysts were identified in 66 patients, in whom 33 (50%) had a single cyst and the rest had 2 or more cysts. The average dose of bevacizumab was 2.68 mg/kg per week. Median duration of treatment was 33 weeks. Average cyst size was 1.9±2.4 cm at the beginning of the study and the majority of the cysts (54 patients, 84%) did not change in size or shape during bevacizumab treatment. No patients were identified with new cysts. Cyst size changed in 10 patients (16%): an increase of 15% to 40% from the baseline size in 5 patients and a decrease in size of 10% to 70% in another 5 patients. The duration of bevacizumab therapy was significantly longer in the subgroup of patients with diminished or increased cyst size than in the patients with stable cyst size: 62 weeks versus 29 weeks, respectively (p=0.0002). CONCLUSIONS: Our data demonstrated that simple renal cysts were stable in size and number in the vast majority of cancer patients treated with bevacizumab.

Cisto assintomático em região da valécula: relato de caso / Asymptomatic vallecular cyst: case report / Quiste valecular asintomático: relato de caso

Paciente do sexo masculino, 56 anos, apresentou-se para excisão de glioblastoma intracraniano multiforme. Após ser rotineiramente monitorado, o paciente foi pré-oxigenado. Anestesia e paralisia foram induzidas com propofol (200 mg), fentanil (50 µg) e vecurônio (9 mg). Laringoscopia direta com lâmina Macintosh número 3 revelou um cisto pedunculado de 2x2 cm, que surgia do lado direito da valécula e impedia a intubação endotraqueal. Enquanto o paciente permanecia anestesiado, consultamos rapidamente um otorrinolaringologista e o cisto foi aspirado por uma seringa com agulha de calibre 22G sob laringoscopia direta. Aspiramos 10 cc de líquido. Intubação traqueal foi feita em seguida sem intercorrências com sonda de 9,0 aramada e com balão. Uma opção para a intubação com fibra óptica pode ser a aspiração cuidadosa do cisto para facilitar a intubação.

A 56-year-old man presented himself for an intracranial glioblastoma multiforme excision. After being routinely monitored, he was preoxygenated. We induced anesthesia and paralysis with 200 mg propofol, 50 µg fentanyl and 9 mg vecuronium. Direct laryngoscopy with a Macintosh 3 blade revealed a 2x2 cm cyst, pedunculated, arising from the right side of the vallecula preventing the endotracheal intubation. While the patient remained anesthetized, we urgently consulted an otolaryngologist and aspirated the cyst with a 22-gauge needle and syringe under direct laryngoscopy. We aspirated 10 cc of liquid content. This was followed by an uneventful tracheal intubation with a 9.0 enforced spiral cuffed tube. An alternative to fiberoptic intubation may be careful cyst aspiration to facilitate the intubation.

Un paciente del sexo masculino, de 56 años, llegó para una resección de glioblastoma intracraneal multiforme. Posteriormente a la rutina de monitorización, el paciente fue pre-oxigenado. La anestesia y la parálisis se indujeron con propofol (200 mg), fentanilo (50 µg) y vecuronio (9 mg). La laringoscopia directa con lámina 3 Macintosh arrojó un quiste pedunculado de 2x2 cm que surgía al lado derecho de la valécula e impedía la intubación endotraqueal. Mientras el paciente permanecía anestesiado, consultamos rápidamente un otorrinolaringólogo y el quiste fue aspirado por una jeringa con una aguja calibre 22G bajo laringoscopia directa. Aspiramos 10 cc de líquido. La intubación traqueal se hizo enseguida sin intercurrencias con una sonda de 9,0 y un alambre en espiral y con balón. Una opción para la intubación con fibra óptica puede ser la aspiración cuidadosa del quiste para facilitar la intubación.

Lung cysts in chronic paracoccidioidomycosis / Cistos pulmonares na paracoccidioidomicose crônica

On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung parenchyma and a well-defined interface with the normal adjacent lung. The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. In a retrospective analysis of the HRCT findings in 50 patients diagnosed with chronic paracoccidioidomycosis, we found lung cysts in 5 cases (10%), indicating that patients with paracoccidioidomycosis can present with lung cysts on HRCT scans. Therefore, paracoccidioidomycosis should be included in the differential diagnosis of cystic lung diseases. .

Os cistos pulmonares na TCAR são caracterizados por áreas arredondadas de baixo coeficiente de atenuação no parênquima pulmonar com uma interface bem definida com o pulmão adjacente normal. As doenças pulmonares císticas mais comuns são linfangioleiomiomatose, histiocitose de células de Langerhans e pneumonia intersticial linfocítica. Em uma análise retrospectiva de achados de TCAR em 50 pacientes com diagnóstico de paracoccidioidomicose crônica residual, observou-se a presença de cistos pulmonares em 5 casos (10%), mostrando que pacientes com paracoccidioidomicose podem apresentar cistos pulmonares na TCAR. Portanto, essa infecção deve entrar no diagnóstico diferencial das doenças císticas pulmonares. .

Quiste congénito de la valécula con alteraciones del peso: Caso clínico / Congenital vallecular cyst with weight disorders: Case report

Los quistes de la valécula son un trastorno raro en los niños; el estridor es el síntoma más frecuente y la alteración del crecimiento, el más infrecuente. Se presenta el caso de un niño de 2 meses referido por la escasa ganancia ponderal desde su nacimiento, con antecedentes de atragantamiento al alimentarse. No presentaba dificultad respiratoria, pero llamaba la atención la respiración bucal y la hiperextensión del cuello con lateralización hacia la derecha. El esofagograma mostró una masa hipofaríngea que desplazaba la laringe y reflujo del medio de contraste hacia el árbol bronquial. Por laringoscopia directa se resecó un quiste con técnica de marsupialización. La evolución fue favorable, sin recidivas, hasta el cuarto mes de seguimiento.

Vallecula cysts are a rare condition in children, with stridor being the most common symptom and growth alterations the more uncommon. We present a 2 months-old child referred by low weight gain from birth, with a history of choking when feeding. He had no respiratory distress but struck mouth breathing and neck hyperextension with lateralization to the right. The esophagogram showed a mass displacing hypopharynx and larynx reflux of contrast into the bronchial tree. The cyst was resected by direct laryngoscopy with marsupialization technique. The outcome was favorable, without recurrence until the fourth month of monitoring.

A Case of Hepatic Peribiliary Cysts in a Patient with Alcoholic Liver Cirrhosis / 대한소화기학회지

Hepatic peribiliary cysts (HPCs) are characterized by cystic dilatations of the peribiliary glands located throughout the branches of the biliary systems. Specifically, they are mainly located along the hepatic hilum and major portal tracts. The natural history and prognosis of HPCs are uncertain. In fact, almost all HPCs have been discovered incidentally during radiological examination or autopsy, and they are considered to be clinically harmless. Recently, several cases of HPCs associated with obstructive jaundice or liver failure were reported in patients with pre-existing liver disease in several studies. However, until now there have been no reports of such a case in Korea. Herein, we report a case of HPCs that show a disease course with a poor prognosis. These HPCs developed in a 47-year-old man with progressive alcoholic liver cirrhosis.

Quiste suprarrenal primario / Primary adrenal cyst

Adrenal cyst is the commonest type of benign lesions of adrenal gland, althought is a very rare entity. Tipically, they are presented with abdominal pain or palpable mass, but nowdays they are detected most frecuently an imaging studies like incidentaloma. Adrenal Cyst have a broad differential diagnoses, rendering definitive diagnosis and subsequent management difficult. Are categorized into four subtypes: endothelial, pseudocyst, epithelial, and parasitic. This management paradigm may be summarized as: ruling out functional status of the cyst, evaluating chances of incidental malignancy by imaging, avoiding potential complications of surveillance (hemorrhage, infection), particularly in large cysts. A case of a patient with a left non-functioning adrenal cyst is reported, with abdominal pain. The diagnostic and therapeutic options are discussed and the literature is reviewed.

Los quistes suprarrenales son las lesiones quísticas más frecuentes de la glándula suprarrenal, a pesar de que constituyen una rara entidad. Típicamente se presentan por un cuadro de dolor abdominal o masa palpable, pero en la actualidad se detectan cada vez con más frecuencia en estudios radiológicos como incidentalomas. Los quistes suprarrenales cuentan con amplios diagnósticos diferenciales, lo que hace un difícil diagnóstico definitivo y una dificultad en el manejo posterior. Estos quistes se clasifican en cuatro subtipos: endoteliales, pseudoquiste, epiteliales y parasitarios. El manejo de un quiste suprarrenal se puede resumir en tres pilares fundamentales: descartar el estado funcional del quiste, evaluación de eventual malignidad por imágenes, y evitar las posibles complicaciones (hemorragia, infección), sobre todo en los quistes de gran tamaño. Se presenta el caso de una paciente con quiste suprarrenal izquierdo no funcionante asociado a dolor abdominal. Se realiza revisión de la literatura y se plantean los distintos diagnósticos y alternativas terapéuticas.

Cystic medial necrosis: pathological findings and clinical implications / Necrose cística da média: manifestações patológicas com implicações clínicas

Cystic medial necrosis (CMN) is a disorder of large arteries, in particular the aorta, characterized by an accumulation of basophilic ground substance in the media with cyst-like lesions. CMN is known to occur in certain connective tissue diseases such as Marfan syndrome, Ehlers-Danlos syndrome, and annuloaortic ectasia, which usually result from degenerative changes in the aortic wall. The relationships between CMN and congenital heart defects as well as other disorders have been evidenced. The mechanisms are still controversial, even though many molecular studies have been conducted. The aim of the present article is to provide a comprehensive overview of the CMN lesion in terms of pathologic features, clinical implications and etiologies based on molecular research results.

A necrose cística da média (NCM) é uma desordem das grandes artérias, em particular a aorta, caracterizada por acúmulo de substância basofílica na camada média com lesões císticas-símile. É sabido que a NCM ocorre em certas doenças do tecido conjuntivo tal como síndrome de Marfan, síndrome de Ehlers-Danlos, e ectasia ânulo-aórtica, que normalmente resulta de mudanças degenerativas na parede aórtica. A relação entre NCM e defeitos congênitos do coração, assim como outras desordens, tem sido evidenciada. Os mecanismos são ainda controversos, embora muitos estudos moleculares tenham sido conduzidos. O objetivo do presente artigo é fornecer uma visão geral da NCM em termos de características patológicas, implicações clínicas e etiologia baseada em resultados de pesquisa molecular.

Van der woude syndrome with an unusual intraoral finding.

Orofacial manifestations of Van der Woude syndrome (VWS) include cleft lip or palate, lower lip pits, hypodontia, hypernasal voice, cleft or bifid uvula, syngnathia, narrow high arched palate, and ankyloglossia. Extraoral manifestations include limb anomalies, popliteal webs, accessory nipples, congenital heart defects, and Hirschsprung disease. We report an interesting case of VWS with characteristic orofacial features along with an unusual additional finding of fusion of primary mandibular left lateral incisor and canine in a 7-year-old boy.

Cisto vítreo e retinose pigmentária: relato de caso / Vitreous cyst and retinitis pigmentosa: case report

Cistos vítreos são achados raros do segmento posterior ocular. Podem ocorrer em olhos com doenças oculares preexistentes ou em olhos aparentemente normais. Este estudo é um relato de caso de um paciente com retinose pigmentária e cisto vítreo, e descreve sua apresentação clínica e ultrassonográfica.

Vitreous cyst is a rare condition of the posterior segment of the eye. It can occur in eyes with coexistent ocular diseases or in eyes that are otherwise normal. This study reports a case of vitreous cyst in a patient with retinitis pigmentosa and presents its clinical and ultrasonographic features.